engleski

OEIS complex - report of two cases

Exstrophy of the cloaca, often referred as OEIS complex (omphalocele, exstrophy, imperforate anus and spinal defects) is the most severe end of the spectrum of the exstrophy-epispadias complex. We report two infants with OEIS complex. Parents are healthy and non-consanguineous. Family history is unremarkable. Surgery was performed on fourth and first day of life respectively. First patient: The postdelivery examination showed cloacal exstrophy with large bladder halves and ureteral orifices bilaterally, terminal ileum protruding between the bladder halves and a small opening of the blind-ending foreshortened hindgut on the left, small and bifid penis with adjacent scrotum on each side. He had an omphalocele and imperforate anus. Renal ultrasound was normal. X-ray revealed hemivertebrae in the thoracic spine and widely separated pubic symphysis. Second patient: It was a twin pregnancy. Prenatal ultrasound revealed one already lost twin, an omphalocele and right hydronephrosis. On examination, there was an cloacal exstrophy, a bifid clitoris and a single perineal opening that drained into a common cloaca with a vaginal halves located on each side. Scintigraphy showed horseshoe kidney and hydronephrosis. X-ray revealed cervical spine alordosis, hypoplastic C4 and lower thoracic and lumbar vertebral malformations. Incidence of the OEIS complex is 0.5–1/200.000 live births. The underlying cause remains unknown. Blood samples were sent for a genetic analysis. Prenatal diagnosis should lead to parental counseling. The aims of surgical management include abdominal wall closure, prevention of short bowel syndrome, provision of fecal and urinary continence, preservation of renal function and reconstruction of functional and cosmetically acceptable genitalia. The success of these procedures cannot guarantee a good quality of life. The survivors have long-term psychosexual developmental difficulties and require life-long follow-up from a multidisciplinary team of experts.

OEIS complex; children

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